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[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Histopathologic grading of adult medulloblastomas.

BACKGROUND: Histopathologic evaluation of the degree and extent of anaplasia is a useful prognostic parameter in pediatric medulloblastomas.

Whether the same applies to adult medulloblastomas is not known.

METHODS: The study included 74 adult patients with histologically confirmed medulloblastomas and retrospectively reassessed 67 cases with available slides for the presence of nodularity, collagen deposition (desmoplasia without nodules), and degree and extent of anaplasia.

CONCLUSIONS: The incidence of severe anaplasia in adult medulloblastomas is lower than in the pediatric population.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] The miR-17/92 polycistron is up-regulated in sonic hedgehog-driven medulloblastomas and induced by N-myc in sonic hedgehog-treated cerebellar neural precursors.

Medulloblastoma is the most common malignant pediatric brain tumor, and mechanisms underlying its development are poorly understood.

We identified recurrent amplification of the miR-17/92 polycistron proto-oncogene in 6% of pediatric medulloblastomas by high-resolution single-nucleotide polymorphism genotyping arrays and subsequent interphase fluorescence in situ hybridization on a human medulloblastoma tissue microarray.

Profiling the expression of 427 mature microRNAs (miRNA) in a series of 90 primary human medulloblastomas revealed that components of the miR-17/92 polycistron are the most highly up-regulated miRNAs in medulloblastoma.

Expression of miR-17/92 was highest in the subgroup of medulloblastomas associated with activation of the sonic hedgehog (Shh) signaling pathway compared with other subgroups of medulloblastoma.

Medulloblastomas in which miR-17/92 was up-regulated also had elevated levels of MYC/MYCN expression.

Consistent with its regulation by Shh, we observed that Shh treatment of primary cerebellar granule neuron precursors (CGNP), proposed cells of origin for the Shh-associated medulloblastomas, resulted in increased miR-17/92 expression.

We conclude that miR-17/92 is a positive effector of Shh-mediated proliferation and that aberrant expression/amplification of this miR confers a growth advantage to medulloblastomas.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

The homeobox transcription factor OTX2 plays an essential role during embryonic brain development.

It is normally silenced in the adultbrain, but is overexpressed by genomic amplification or other mechanisms in the majority of medulloblastomas (MBs).

These findings suggest a mechanism for RA-mediated anti-tumor effect on OTX2-positive MB cells and indicate that therapeutic targeting of OTX2 might be effective if FGF pathway-mediated resistance can be overcome.

Significant differences were found in the patterns of copy number change between medulloblastomas and supratentorial primitive neuroectodermal tumors, providing further evidence that these tumors are genetically distinct despite their morphologic and behavioral similarities.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Subtype-specific expression and genetic alterations of the chemokinereceptor gene CXCR4 in medulloblastomas.

As medulloblastomas (MBs), the most common malignant brain tumors of childhood, are believed to arise from neuronal cerebellar precursors, we asked whether there is a potential role for Cxcr4 in the pathogenesis of MB.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Morphophenotype of medulloblastoma in children and adults. The size of nuclei.

Enlargement of nuclei distinguishes the large-cell medulloblastoma variant and is associated with a poor prognosis in pediatric medulloblastomas.

The aim of the present study was to compare the size of nuclei between pediatric and adult medulloblastomas by a morphometric analysis.

MATERIAL AND METHODS: In 79 neurosurgical specimens of cerebellar medulloblastomas, the maximum nuclear diameter of the largest nuclei was measured.

Measurements were performed with a digital-image analysis system.

RESULTS: The difference between the mean values in children and adults was statistically significant (p = 0,001).

The distribution of maximum values measured in each case had two distinct peaks in the two age groups, in 3.5% of adult cases and in more than 30% of pediatric cases the maximum nuclear size was superior to 12 microm.

CONCLUSIONS: The present results show that nuclei of tumor cells in pediatric medulloblastomas are larger than those in adult medulloblastomas and confirm that the phenotype of medulloblastoma is different in the two age groups.

Distinct genetic events can, thus, underlie medulloblastoma in childhood and adult age, the prognostic role of genetic variables can differ by age.

We performed comprehensive cytogenetic analyses of an adultmedulloblastoma, WHO grade IV, using trypsin-Giemsa staining (GTG-banding), multicolor fluorescence in situ hybridization (M-FISH), and locus-specific FISH, complemented by molecular karyotyping using high-density single nucleotide polymorphism (SNP) arrays.

Applying an upcoming therapeutic approach, we found that primary medulloblastoma cells were resistant to TRAIL, a novel anticancer cytokine, but could be efficiently sensitized by cotreatment with the proteasome inhibitor bortezomib.

Bortezomib-TRAIL cotreatment may serve as a powerful therapeutic option for medulloblastoma patients.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

: 2030 Background: While in children medulloblastoma comprises the most common malignant brain tumor, it accounts for only 1% of intracranial malignancies in adults.

The infrequent appearance of MB in adults poses the question, whether these tumors are the same in adults and children in terms of biological and clinical peculiarities.

METHODS: Array-CGH was performed for a total 34 adultmedulloblastoma samples (>18 years) and results were compared with data from 101 pediatric patients.

Selected genomic regions were further investigated by FISH analysis in an independent cohort of 415 samples (112 adult and 303 pediatric).

All 146 adult patients received a standard treatment regimen consisting of tumor resection, irradiation of the neuroaxis with 36 Gy, a boost of 20-23 Gy to the posterior fossa, and eight cycles of vincristin, lomustine, and cisplatin.

RESULTS: Copy-number gains of chromosome 17q as well as high-level amplifications of CDK6 were identified as significant adverse prognostic markers in adultmedulloblastoma.

Apart from one exception, CDK6 amplifications were only observed in adult patients (9% in adults versus 0.2 % in children), whereas amplifications of MYC or MYCN were significantly overrepresented in the pediatric cohort, but when present were also associated with dismal prognosis in adults.

Based on these results, we propose a molecular staging system for adultmedulloblastoma: i) cases with oncogene amplification (10% of cases, 5-year OS = 0%);.

CONCLUSIONS: We report on the largest cohort of adultmedulloblastoma investigated for genomic imbalances to date.

We propose a model for the molecular risk stratification of adultmedulloblastoma comprising three distinct genomic risk groups with significantly different survival and tumor biology.

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(PMID = 27964634.001).

[ISSN] 1527-7755

[Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology

The OTX2 gene was amplified in the medulloblastoma cell line D425 and mRNA and protein data showed expression in 114 of 152 medulloblastomas (75%), but not in postnatal cerebellum.

OTX2 mRNA expression correlated with a classic medulloblastoma histology (29 of 34 cases), whereas expression of OTX1 mRNA only was correlated with a nodular/desmoplastic histology (9 of 11 cases).

Immunohistochemical analysis of a series of classic medulloblastomas detected OTX2 protein expression in 83 of 107 (78%) cases.

In addition, OTX2-positive tumors were mainly found in children, but OTX2-negative tumors occurred in 2 patient groups: very young patients (<5 years) and adults (>20 years).

Nodular/desmoplastic medulloblastomas are thought to arise from the external granular layer (EGL).

However, it is unclear whether classic medulloblastomas also originate from the EGL or from the ventricular matrix.

Analysis of human fetal brain showed OTX2 protein expression in a small number of presumptive neuronal precursor cells of the EGL, but not in precursor cells of the ventricular matrix.

Combined with data from rodents, our results therefore suggest that both nodular/desmoplastic and at least part of the classic medulloblastomas originate from cells of the EGL, albeit from different regions.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Correlation of gamma-catenin expression with good prognosis in medulloblastomas.

OBJECT: Medulloblastoma is a malignant cerebellar tumor of childhood and is difficult to cure due to frequent cerebrospinal fluid dissemination.

METHODS: Immunohistochemical and cytogenetic examinations of beta- and gamma-catenin, c-myc, N-myc, and cyclin D1 in 24 primary medulloblastomas were conducted, and their clinical relevance was evaluated.

By differential polymerase chain reaction, c-myc and N-myc were amplified separately in two large cell/anaplastic medulloblastomas.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

Medulloblastoma is the most common malignant pediatric central nervous system tumor.

Despite the adequate therapy the tumor often recurs.

The primary medulloblastoma expresses somatostatin receptor-2 (SSTR-2), but so far we had no experience about the receptor status in recurrent tumors.

The presence of SSTR-2 may have an important role in the early detection and treatment of recurrent medulloblastomas.

Our aim was to examine the state of SSTR-2 expression in recurrent childhood medulloblastomas.

We examined SSTR-2 expression by immunohistochemistry in primary and recurrent medulloblastoma samples of ten children treated with recurrent medulloblastoma at Semmelweis University, Departments of Pediatrics, between 1998 and 2004.

We examined the intensity and the percentage of SSTR-2-positive tumor cells in the primary and recurrent tumor samples.

All primary tumors were receptor-positive and SSTR-2 was also expressed in all recurrent medulloblastomas.

In our samples the percentage of SSTR-2-positive tumor cells was 30-90%.

As a conclusion, SSTR-2-positive recurrent tumors can be detected early by Octreoscan imaging, and the presence of SSTR-2 establishes the opportunity of applying somatostatin analogues (octreotide) in the treatment of recurrent childhood medulloblastoma.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Rarity of JC virus DNA sequences and early proteins in human gliomas and medulloblastomas: the controversial role of JC virus in human neurooncogenesis.

Moreover, JCV genomic DNA and early viral protein T-antigen have been detected in various types of human central nervous system (CNS) neoplasms.

To further explore this association we have studied paraffin-embedded brain biopsy tissue from 60 neoplasms (55 gliomas and five medulloblastomas) and 15 reactive gliosis cases for the presence of JCV DNA sequences and proteins.

Additionally, IHC with both antibodies was applied to a tissue micro-array including 109 CNS tumours and 21 reactive gliosis samples.

The rarity of JCV DNA sequences and early proteins in our brain tumours enriches the controversy over the role of JCV in human neurooncogenesis, whose clarification is in need of further molecular and epidemiologic studies.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

OBJECT: The objective of this study was to determine the role of intracranial CSF examination in detecting true cases of early tumor dissemination.

Cerebrospinal fluid dissemination is an ominous feature of pediatric brain tumors, occurring in as many as 30% of medulloblastomas, 25% of supratentorial primitive neuroectodermal tumors (PNETs), and 5% of ependymomas at diagnosis.

METHODS: Under an institutional review board-approved protocol, medical records, pathology reports, and radiology reports for 150 patients who had undergone resection of brain tumors (88 with medulloblastomas, 21 with supratentorial PNETs, and 41 with ependymomas) and who had been evaluated using neuraxis MR imaging studies in the last 15 years were retrospectively reviewed.

CONCLUSIONS: Discordance exists between the results of neuraxis MR imaging and lumbar and intracranial CSF cytology in perioperative detection of tumor dissemination for pediatric medulloblastoma, supratentorial PNETs, and ependymoma.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

OBJECT: The aim of this paper was to determine prognostic factors for adultmedulloblastoma treated with boost Gamma Knife surgery (GKS) following resection and craniospinal irradiation.

METHODS: The authors performed a retrospective analysis of 12 adult patients with histologically proven medulloblastoma or supratentorial primitive neuroectodermal tumor who between February 1991 and December 2004 underwent >or=1 sessions of GKS for posttreatment residual or recurrent tumors (6 tumors in each group).

Stereotactic radiosurgery was applied to residual and recurrent posterior fossa tumor as well as to foci of intracranial medulloblastoma metastases.

The mean GKS-treated tumor volume was 9.4 cm3 (range 0.5-39 cm3).

RESULTS: Following adjunctive radiosurgery, 5 patients had no evidence of tumor on magnetic resonance (MR) imaging, 3 patients had stable tumor burden on MR imaging, and 4 patients had evidence of tumor progression locally with or without intracranial metastases.

All patients with tumor progression died.

The majority of patients who achieved tumor eradication (80%) and tumor stabilization (67%) after GKS had residual tumor as the reason for their referral for GKS.

The best outcomes were attained in patients with residual disease who were younger, had smaller tumor volumes, had no evidence of metastatic disease, and had received higher cumulative GKS doses.

CONCLUSIONS: Single or multiple GKS sessions were a well-tolerated, feasible, and effective adjunctive treatment for posterior fossa residual or recurrent medulloblastoma as well as intracranial metastatic medulloblastoma in adult patients.

The proportion of phosphorylated STAT3alpha relative to STAT3alpha was significantly greater in supratentorial PNETs than in medulloblastomas, indicating activation of the JAK/STAT3 pathway in supratentorial PNETs.

CONCLUSIONS: These results indicate that supratentorial PNET predominantly has glial features and medulloblastoma largely follows a neuronal differentiation pattern.

These divergent differentiation patterns may be related to the location and origin of each tumor.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] [Chromosome 17 abnormalities in medulloblastomas and their prognostic value].

The interphasic cytogenetic analysis of chromosome 17 abnormalities in medulloblastoma biopsy specimens may be recommended for its inclusion into a complex of laboratory diagnostic methods used in the examination of these tumors.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

The current study was undertaken to examine the mRNA expression of survivin isoforms and their correlation with clinical staging and outcome in 20 medulloblastoma (MB) tumours, three MB cell lines and normal brain tissues (a foetal and an adult cerebellum) by densitometry scanning of 32p-dCTP incorporated reverse transcription polymerase chain reaction (RT-PCR) products and quantitative real-time PCR.

Our results showed that the normal adultbrain only expressed low levels of survivin-deltaEx3 mRNA, while the foetal brain expressed all three isoforms, with wild-type survivin as the dominant transcript.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Expression of p75NTR in fetal brain and medulloblastomas: evidence of a precursor cell marker and its persistence in neoplasia.

p75 neurotrophin receptor (p75NTR) is a member of the tumor necrosis factor superfamily, and plays a significant role in nervous system development. p75NTR has a dual (proliferative/apoptotic) role in neurogenesis and binds pro-neurotrophins with high affinity.

Recent work suggests p75NTR is overexpressed in the developing cerebellum and in nodular/desmoplastic medulloblastomas.

We analyzed p75NTR expression in various parts of the fetal and adult human central nervous system, and in 75 patients with medulloblastomas.

The expression of p75NTR in the fetal brain was seen solely within the external granular layer with weaker expression in the Purkinje layer, which most likely represents Purkinje cell staining.

The staining was present in gestational weeks 20-40, while no staining was identified elsewhere in the fetal brain or within the adult cerebellum. p75NTR positive cells were also positive with the proliferation marker ki-67, but were negative for ret, reelin, CD133, CD34, and cleaved caspase 3.

Nine of 75 medulloblastomas (12%) were also showed positive immunostaining for p75NTR.

The staining was seen in four classic, two desmoplastic, and three anaplastic medulloblastomas.

The persistence of p75NTR in a small group of medulloblastomas raises the possibility that in such tumors, the receptor could be a potential therapeutic target.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Survival and prognostic factors in a series of adults with medulloblastomas.

OBJECT: In this article, the authors report their experience in the management of adult patients with medulloblastoma at their institution to identify prognostic factors important for survival and disease control.

METHODS: Between 1977 and 2005, 27 patients who were >or=16 years old and had medulloblastoma were treated consecutively.

Six patients had the desmoplastic variant, and 21 patients presented with classic medulloblastoma.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Correlation between pre- or postoperative MRI findings and cerebellar sequelae in patients with medulloblastomas.

INTRODUCTION: Immediate and delayed cerebellar dysfunction may be expected after surgical resection of a medulloblastoma.

MATERIAL AND METHODS: The data of 31 patients in continuous complete remission after removal of medulloblastoma, irradiation and chemotherapy, were retrospectively reviewed.

Magnetic Resonance Imaging (MRI) was analyzed for the following items: preoperative MRI (ratio of the surface of the tumor/posterior fossa, presence of ventricular dilatation or tonsilar hernia, involvement of the dentate nucleus) and delayed post-operative MRI (amount of cerebellar parenchyma removed, degree of cerebellar atrophy, presence of T1 hypointense regions in remaining cerebellar area and removal of region containing dentate nucleus).

RESULTS: On preoperative MRI, the ratio of the surface of the tumor/posterior fossa and the presence of tonsilar hernia were significantly correlated with long-term sequelae on speech (respectively P = 0.027 and P = 0.05).

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] [Childhood and adultmedulloblastoma: what difference?].

Medulloblastoma is the most frequent childhood brain tumor (30%) but account only for less than 1% of adultbrain tumor.

Due to the rarety in adult population, no prospective studies and few data about late effects are available.

Adultmedulloblastoma is a therapeutic challenge and their therapeutic strategies are similar to pediatric protocols.

In order to improve the understanding of adult disease and to homogenize the treatment, National Cancer Institute (INCa) stimulated the creation of web conference to discuss each case prospectively and to propose a protocol of treatment.

A better comprehension of biological processes and abnormal cellular signalling pathways involved in medulloblastoma pathogenesis had led toward a new prognostic classification to adapt the therapeutic strategy and gives hope of new therapeutic tools.

Evidence provided in this paper confirms previous reports in the pediatric population and suggests that neuroradiologist and medical oncologists should be aware of new possible radiological findings related to aggressive treatments for brain tumors.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] The tumour-associated carbonic anhydrases CA II, CA IX and CA XII in a group of medulloblastomas and supratentorial primitive neuroectodermal tumours: an association of CA IX with poor prognosis.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title]Medulloblastomas: a correlative study of MIB-1 proliferation index along with expression of c-Myc, ERBB2, and anti-apoptotic proteins along with histological typing and clinical outcome.

BACKGROUND: Medulloblastoma (MB) is the most common pediatric brain tumor.

It is however rare in adults.

The genetic and protein expression profile of medulloblastoma is complex, which is worthwhile in terms of prognostication and development or selection of targeted therapy.

AIMS AND OBJECTIVES: The aims and objectives to correlate the MIB-1 proliferation index and protein expression profiles of c-Myc, ERBB2, and anti-apoptotic proteins (Bcl2 and Bcl-xL) in tumor cells with histological subtypes and clinical outcome.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Radiation induced adultmedulloblastoma: a case report.

Adultmedulloblastoma is a rare intracranial tumor.

Our patient is a 61 year old woman treated with cranial irradiation 15 years previously for a low grade astrocytoma in the left posterior temporal lobe that was recently diagnosed with medulloblastoma in the right cerebellum.

This is the first reported case of radiation induced adultmedulloblastoma.

Human parathyroid hormone-related protein (hPTHrP), identified in patients with paraneoplastic hypercalcemia and expressed by different cell types during development and adult life, plays important roles in many human neoplasms.

Immunohistochemical and RT-PCR analyses of hPTHrP and human parathyroid hormone receptor type 1 (PTHR-1) in primary medulloblastoma confirmed their expression in both classic and desmoplastic variants at RNA and protein levels.

To evaluate the functional role of hPTHrP, DAOY and D283 medulloblastoma and U87MG glioma cells, expressing high levels of hPTHrP and PTHR-1, were treated with anti-sense oligonucleotides for hPTHrP.

This study indicates that hPTHrP and PTHR-1 are expressed in medulloblastoma and could promote tumor growth, protecting cells from apoptosis.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Antiangiogenic therapy in the treatment of recurrent medulloblastoma in the adult: case report and review of the literature.

Medulloblastoma is a rare tumor in central nervous system, with an even rarer occurrence in adulthood.

We report the case of a 51-year-old man with recurrent medulloblastoma.

The aim of this report is to show that recurrent medulloblastoma in adults can be approached with a multimodality treatment and that antiangiogenic therapy should have a role in the management of this disease.

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The imaging pattern is well studied in the pediatric group thought there is controversy about the imaging characteristics in adults.

The imaging findings of medulloblastomas in adults are unspecific and different from those in child.

They should be considered in the differential diagnosis of cerebellar tumor in adults, especially if they are hyperdense on CT, with well defined margins, with superficial extension and with dural involvement.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Molecular pathogenesis of adultbrain tumors and the role of stem cells.

The genetic and signaling abnormalities involved in tumor initiation and progression of the most prevalent adult primary brain tumors, including gliomas, meningiomas, and medulloblastomas, are described in this article.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

Lhermitte-Duclos disease, also known as dysplastic cerebellar gangliocytoma, is a rare cerebellar benign tumor with characteristic appearance of thickened cerebellar folia giving a laminated or striated appearance, quite diagnostic of the condition.

We had seen a patient with medulloblastoma with imaging findings suspicious for thickened cerebellar folia reminiscent of Lhermitte-Duclos disease.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Long-term results of a prospective study on the treatment of medulloblastoma in adults.

BACKGROUND: Because medulloblastoma (MB) is rare in adults, the few studies on this condition have been retrospective, and the follow-up has tended to be short.

METHODS: In 1989, a prospective Phase II trial was initiated to evaluate the efficacy of treatment for adults with MB.

Patients were staged completely with a neuroradiologic examination of the brain and neuroaxis and by cerebrospinal fluid cytology, according to Chang's staging system.

RESULTS: After a median follow up of 7.6 years, among a total of 36 adults with MB, the overall progression-free survival (PFS) and overall survival (OS) rates at 5 years were 72% and 75%, respectively.

CONCLUSIONS: In adult patients with MB, long-term follow-up was essential for evaluating the real impact of treatments.

[Title] MATH-1 production by an adultmedulloblastoma suggestive of a cerebellar external granule cell precursor origin.

Postoperative neuronal imaging studies showed that the tumor located in the cerebellar folia had been removed totally.

Pathological examination identified it as a desmoplastic medulloblastoma with subpial and subarachnoid infiltration and some infiltration into the molecular and granular layer via the perivascular space.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

Gliomas and medulloblastomas are the most frequent malignant brain tumors in adult and children respectively.

Although both tumors arise in the CNS, there is a significant difference in their therapeutic response.

Medulloblastomas are relatively curable, while glioblastomas are basically incurable.

During the last decade several reports have demonstrated the existence of cancer stem cells in brain tumors, their location and their response to treatment.

We have recently described the therapeutic response of medulloblastomas to radiation in their native microenvironment, illustrating how p53 and Pi3K signaling pathways lead to the evasion of cell death by the nestin-expressing cells in the perivascular stem cell niche, even while the bulk of tumor succumbs to apoptosis.(1) It remains to be determined whether this mechanism of tumor resistance applies to the more complex stem-cell niche and tumor bulk of gliomas.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Prognostic significance of apoptosis in medulloblastoma.

Since apoptosis is a major contributor to cell loss in medulloblastoma, either spontaneous or induced by radiation and chemotherapy, the apoptotic rate in resection specimens could have prognostic significance.

We analysed the apoptotic rate in 58 medulloblastoma resection specimens using an antibody against cleaved caspase 3, a specific marker of apoptotic cell death, and tested its possible prognostic significance.

The apoptotic rate was higher in medulloblastomas with CSF dissemination, tended to be higher in desmoplastic medulloblastomas, but there was no association with age group and sex.

The variation in apoptotic rate among medulloblastomas is very likely predominantly associated with variations in tumour microenvironment, as supported by apoptotic cell clustering and rimming around necrotic areas.

The apoptotic rate in medulloblastoma resection specimens does not seem to be of prognostic value.

EXPERIMENTAL DESIGN: Ap endo activity was assayed in 52 medulloblastomas and 10 PNETs from patients 0.4 to 21 years old.

Cox proportional hazards regression models were used to analyze the association of activity with time to tumor progression (TTP).

RESULTS: Tumor Ap endo activity varied 180-fold and was significantly associated with age and gender.

Tumor Ape1/Ref-1 was detected almost exclusively in nuclei.

In a multivariate model, with Ap endo activity entered as a continuous variable, the hazard ratio for progression after adjuvant treatment in 46 medulloblastomas and four PNETs increased by a factor of 1.073 for every 0.01 unit increase in activity (P < or = 0.001) and was independent of age and gender.

Suppressing Ap endo activity in a human medulloblastoma cell line significantly increased sensitivity to 1,3-bis(2-chlororethyl)-1-nitrosourea and temozolomide, suggesting that the association of tumor activity with TTP reflected, at least in part, abasic site repair.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] MR signal of the solid portion of pilocytic astrocytoma on T2-weighted images: is it useful for differentiation from medulloblastoma?

BACKGROUND AND PURPOSE: Although imaging features of cerebellar pilocytic astrocytoma and medulloblastoma have been described in many texts, original comparisons of magnetic resonance intensity between these two tumours are limited.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

Their expression has been studied in a wide range of human tumors in adults.

We measured the expression of 12 CGGs in pediatric brain tumors, to identify targets for therapeutic cancer vaccines.

Real Time PCR was used to quantify the expression of genes MAGE-A1, MAGE-A2, MAGE-A3, MAGE-A4, MAGE-A6, MAGE-A10, MAGE-A12, MAGE-C2, NY-ESO-1 and GAGE-1,2,8 in 50 pediatric brain tumors of different histological subtypes.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

Isodicentric 17q is the most commonly reported chromosomal abnormality in medulloblastomas.

Its frequency suggests that genes disrupted in medulloblastoma formation may play a role in tumorigenesis.

CGH with a custom tiling path genomic BAC array of chromosome 17 enriched with fosmids at the breakpoint regions was used to analyze a series of 45 medulloblastomas and three medulloblastoma-derived cell lines.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

OBJECTIVE: To detect and analysis epidermal growth factor receptor (EGFR) and phosphatase and tensin homolog deleted on chromosome ten (PTEN) in different malignancy brain tumors, and to evaluate their prognostic significance.

METHODS: Using immunohistochemistry to detect the expression of EGFR and PTEN and adopting confocal technology to verify their location in the specimens of 25 respectively glioblastoma multiformes, medulloblastomas, anaplastic oligodendrogliomas, and anaplastic ependymomas.

However amplification of EGFR and deletion of PTEN were relatively low in other malignancy brain tumors.

They were 36% and 8% in medulloblastomas, and 28% and 8% in anaplastic oligodendrogliomas, and 24% and 4% in anaplastic ependymomas.

PTEN mutation and EGFR overexpression are rare in medulloblastoma, anaplastic oligodendroglioma, and anaplastic ependymoma, so the EGFR or PTEN targeted antitumor approaches may be useful in glioblastoma multiformes but the other 3 tumors.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

Medulloblastoma encompasses a collection of clinically and molecularly diverse tumour subtypes that together comprise the most common malignant childhood brain tumour.

The pathological processes that drive heterogeneity among the other medulloblastoma subtypes are not known, hindering the development of much needed new therapies.

Here we provide evidence that a discrete subtype of medulloblastoma that contains activating mutations in the WNT pathway effector CTNNB1 (hereafter, WNT subtype) arises outside the cerebellum from cells of the dorsal brainstem.

We found that genes marking human WNT-subtype medulloblastomas are more frequently expressed in the lower rhombic lip (LRL) and embryonic dorsal brainstem than in the upper rhombic lip (URL) and developing cerebellum.

These lesions persisted in all mutant adult mice; moreover, in 15% of cases in which Tp53 was concurrently deleted, they progressed to form medulloblastomas that recapitulated the anatomy and gene expression profiles of human WNT-subtype medulloblastoma.

We provide the first evidence, to our knowledge, that subtypes of medulloblastoma have distinct cellular origins.

Our data provide an explanation for the marked molecular and clinical differences between SHH- and WNT-subtype medulloblastomas and have profound implications for future research and treatment of this important childhood cancer.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

Compared with the previous studies in the United States, the United Kingdom, and Australia, Japanese Gorlin syndrome patients showed a significantly lower rate of BCCs, and no medulloblastomas in this study.

Furthermore, we show that there is loss of physiological KLF4 expression in more than 40% of primary medulloblastomas both at the RNA and protein levels.

Medulloblastoma cell lines drastically increase the expression of KLF4 in response to the demethylating agent 5-azacytidine and demonstrate dense methylation of the promoter CpG island by bisulfite sequencing.

Reexpression of KLF4 in the D283 medulloblastoma cell line results in significant growth suppression both in vitro and in vivo.

We conclude that KLF4 is inactivated by either genetic or epigenetic mechanisms in a large subset of medulloblastomas and that it likely functions as a tumor suppressor gene in the pathogenesis of medulloblastoma.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Survivin is a negative prognostic marker in medulloblastoma.

Our aim was to analyse survivin expression in medulloblastoma, its association with aberrant activation of the WNT (wingless) pathway and to test the prognostic significance of survivin expression.

We immuno histochemically analysed survivin expression and localization of beta-catenin, a downstream mediator of the WNT pathway, in 56 cases of medulloblastoma.

Survivin expression tended to be higher in medulloblastomas with an aberrant activation of the WNT pathway (nuclear localization of beta-catenin), but did not correlate with histological type, age group or dissemination via cerebrospinal fluid pathways.

Survivin expression and dissemination status were two independent negative prognostic variables for the overall survival of patients with medulloblastoma.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Survival of adults treated for medulloblastoma using paediatric protocols.

Although the number of patients is limited, our data suggest that the sandwich sequential, moderately intensive chemotherapy in combination with HART is an effective treatment for medulloblastoma in adults, and this approach seems to overcome previously-recognised risk factors.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Does c-erbB-2 expression have a role in medulloblastoma prognosis?

The prognosis of patients with medulloblastoma has remained same for the last two decades.

This study evaluated the role of c-erbB-2 expression in medulloblastoma as a prognostic marker.

Fifty cases of medulloblastomas were investigated for the expression of c-erbB-2 protein using immunohistochemistry.

The mean DFS in c-erbB-2 positive cases was 19.81 months compared to 48.33 months in c-erbB-2 negative cases. c-erbB-2 positivity was found to be an independent predictor of poor outcome in medulloblastoma (p value < 0.05).

[Other-IDs] NLM/ NIHMS77932; NLM/ PMC2674531

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title]Brain tumor stem cells.

The dogma that solid tumors are composed of tumor cells that all share the same ability to produce proliferating daughter cells has been challenged in recent years.

There is growing evidence that many adult tissues contain a set of tissue stem cells, which might undergo malignant transformation while retaining their stem cell characteristics.

Brain tumors such as medulloblastomas or glioblastomas often contain areas of divergent differentiation, which raises the intriguing question of whether these tumors could derive from neural stem cells (NSCs).This chapter reviews the current knowledge of NSCs and relates them to brain tumor pathology.

Current therapy protocols for malignant brain tumors are targeted toward the reduction of bulk tumor mass.

The concept of brain-tumor stem cells could provide new insights for future therapies, if the capacity for self-renewal of tumor cells and growth of the tumor mass would reside within a small subset of cancer cells.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Metabotropic glutamate receptors: new targets for the control of tumor growth?

Transformed neural stem-progenitor cells of the brain subventricular zone and the external granular layer of the cerebellum are the putative cells of origin of malignant gliomas and medulloblastomas, which are the most frequent malignant brain tumors in adults and children, respectively.

The proliferation of neural stem-progenitor cells is regulated by metabotropic glutamate (mGlu) receptors, which are G-protein-coupled receptors that are activated by glutamate, the major excitatory neurotransmitter of the CNS.

At least two receptor subtypes - mGlu(3) and mGlu(4) receptors - control the proliferation of brain tumor cells, whereas mGlu(1) receptors have been implicated in the development of melanomas.

We believe that individual mGlu receptor subtypes represent new potential targets for the treatment of several malignant tumors, including brain tumors.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

Clinical application of Magnetic Resonance Imaging (MRI) and Computerized Tomography has provided an earlier detection and treatment of many CNS pathologies.

Fifty-nine of them were found to have tumor localized in fossa crani posterior (FCP) without any significant difference between genders (50.8% female vs. 49.2% male, chi2 test=0.02 p=0.896).

Tumor types that more often were found in young's individuals were: Astrocytomas with a peak incidence in teenagers (average age was 12-year-old SD +/- 7.5, rank 3-23), next was Medulloblastomas (average age was 11-years-old, SD +/- 2.9, rank 6-16 years) and ependymomas (average age was 6.8-years-old, SD +/- 4.6, rank 1-12).

The most frequent tumors in children were medulloblastomas, brainstem gliomas, astrocytomas and ependymomas whereas meningiomas and metastasis were most often found in adults.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Sox2 expression in brain tumors: a reflection of the neuroglial differentiation pathway.

Moreover, it was recently found that brain tumors contain stem cells that resemble normal neuroglial stem cells in many respects.

This study was undertaken to describe Sox2 expression in various brain tumors, and to determine whether Sox2 expression is a universal feature of brain tumors, or whether its expression is limited to a specific lineage of brain tumors.

Sox2 immunohistochemistry was performed on 194 brain tumor tissues of various kinds.

Fetal and adult normal brain tissues obtained by autopsy and brain tissues of epilepsy patients with cortical dysplasia were used as controls.

This study suggest that Sox2 may be a tumor marker of glial lineages rather than a universal brain tumor stem cell marker, because its expression pattern was found to correspond to differentiation pathways.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Epigenetic deregulation of multiple S100 gene family members by differential hypomethylation and hypermethylation events in medulloblastoma.

Using a pharmacological expression reactivation approach, we screened 16 S100 genes for evidence of epigenetic regulation in medulloblastoma, the most common malignant brain tumour of childhood.

Four family members (S100A2, S100A4, S100A6 and S100A10) demonstrated evidence of upregulated expression in multiple medulloblastoma cell lines, following treatment with the DNA methyltransferase inhibitor, 5'-aza-2'-deoxycytidine.

Assessment of these genes in the non-neoplastic cerebellum (from which medulloblastomas develop) revealed strong somatic methylation affecting S100A2 and S100A4, whereas S100A6 and S100A10 were unmethylated.

Assessed against these normal tissue-specific methylation states, S100A6 and S100A10 demonstrated tumour-specific hypermethylation in medulloblastoma primary tumours (5 out of 40 and 4 out of 35, respectively, both 12%) and cell lines (both 7 out of 9, 78%), which was associated with their transcriptional silencing.

In summary, these data characterise complex patterns of somatic methylation affecting S100 genes in the normal cerebellum and demonstrate their disruption causing epigenetic deregulation of multiple S100 family members in medulloblastoma development.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

The ependymoma is the second most common malignant brain tumor of childhood; however, its molecular basis is poorly understood.

The formation of multiple ependymomas has been reported as an occasional feature of Turcot syndrome type 2 (TS2), a familial cancer syndrome caused by inherited mutations of the APC tumor suppressor gene, and characterised by the concurrence of a primary CNS tumor (predominantly medulloblastoma) and multiple colorectal adenomas.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] [The medulloblastoma].

INTRODUCTION AND DEVELOPMENT: Medulloblastoma is a cerebellar small cell tumor, whose ancestor cell has not been yet identified in the human normal embriology: its exact origin is, in fact, still unknown.

Nevertheless, one of the most acceptable possibilities facing the origin of the tumor is the remaining rests of cerebellar outer granular sheet.

It is a predominantly infantile tumor, less frequent in young adults, and World Health Organization (WHO) classification has assignated grade IV of malignancy.

In this publication of the WHO, medulloblastomas have been subclassified into: classic, desmoplastic, medulloblastomas with extensive nodularity and advanced neuronal differentiation and large cell medulloblastomas.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Cell cycle times of short-term cultures of brain cancers as predictors of survival.

Brain tumour material obtained at surgery from 70 patients with glioblastoma, medulloblastoma, astrocytoma, oligodendroglioma and metastatic melanoma was cultured for 7 days on 96-well plates, coated with agarose to prevent proliferation of fibroblasts.

For patients with brain cancers of all types, median survival for the < or =10-day and >10-day groups were 5.1 and 12.5 months, respectively (P=0.0009).

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Accumulation of genomic aberrations during clinical progression of medulloblastoma.

Medulloblastomas comprise the most frequent malignant brain tumor in childhood and one of the biggest challenges in pediatric oncology.

The current concept suggests that these tumors may undergo stepwise progression as it has been shown for other brain tumors.

However, conclusive evidence of molecular progression over time has not been demonstrated yet for medulloblastoma.

In the present study, 28 pairs of medulloblastoma at primary diagnosis and at the time of recurrence, either occurring as local tumor regrowth or tumor dissemination, were histopathologically and molecularly analyzed.

These results suggest that early recurrence in medulloblastoma mainly occurs in tumors with a highly malignant genotype and phenotype per se, whereas late recurrence is often dependent on tumor evolution toward a more malignant biology.

Therefore, biopsy of recurrent tumors should be performed to assess the biologic properties of the relapsed tumor, especially when targeted therapy approaches are considered.

The aim of our study was to determine the prevalence of genetic polymorphisms (codon 31 and 3' untranslated region, 3'UTR) and protein expression of the cyclin-dependent kinase inhibitor 1A (CDKN1A) gene in patients with and without CNS tumors.

METHODS: 41 patients with CNS tumors and a control group of 161 subjects without cancer and paires for sex, age and ethnicity were genotyped using polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP).

Protein analysis was performed on 36 patients with CNS tumors, using the Western Blotting technique.

These frequencies were not statistically different (P > 0.05) from those seen in the patients with CNS tumors (19.4% and 0.0%, codon 31; 15.8% and 2.6%, 3'UTR site).

The results for medulloblastomas and astrocytomas were similar: neither of them expressed the protein (57.14% and 61.54%, respectively).

CONCLUSION: No significant differences in protein expression patterns or polymorphisms of CDKN1A in relation to the three types of CNS tumors were observed among Brazilian subjects.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

Supratentorial primitive neuroectodermal tumors (SPNETs) and medulloblastomas (MBs) are histologically similar intracranial tumors found in different anatomic locations of the brain.

The aim of this study was to evaluate whether DLC-1, a newly identified tumor-suppressor gene on chromosome 8p22, is involved in the tumorigenesis of MBs and the histologically similar SPNETs.

Bisulfite sequencing further verified a densely methylated pattern of 35 CpG sites studied in M1 that were not found in normal brain, indicating that inactivation of DLC-1 by hypermethylation is involved in SPNET.

Due to this rarity, few prospective clinical trials have been conducted on medulloblastoma in adults, investigations being based exclusively on retrospective studies; the populations considered in literature are small, and the different treatments given span decades, during which diagnostic procedures, neurosurgical skills and radiotherapy techniques have changed.

Unlike pediatric patients, adultmedulloblastoma patients have been treated according to risk-adapted therapeutic strategies in only a few series and despite risk-tailored treatments, 20-30% of patients experience recurrence.

An important challenge for the future will be the biological characterization of medulloblastoma, with the identification of specific genetic patterns of patients with a better or a worse prognosis.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] MicroRNA profiling in human medulloblastoma.

Medulloblastoma is an aggressive brain malignancy with high incidence in childhood.

However, no data are yet available on human primary medulloblastomas.

A high throughput microRNA expression profiles was performed in human primary medulloblastoma specimens to investigate microRNA involvement in medulloblastoma carcinogenesis.

We identified specific microRNA expression patterns which distinguish medulloblastoma differing in histotypes (anaplastic, classic and desmoplastic), in molecular features (ErbB2 or c-Myc overexpressing tumors) and in disease-risk stratification.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

A 23-year-old woman with basal cell nevus syndrome (BCNS), or Gorlin's syndrome, was given the diagnosis at age 2 years of a medulloblastoma that was resected and treated postoperatively with craniospinal irradiation.

This case reviews early presentation of BCNS, newly described differences between medulloblastomas in patients with BCNS and nonsyndromic medulloblastomas, and global assessment of patients by the treating dermatologist of this patient population.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

To evaluate whether the chemosensitivity of primary central nervous system lymphomas to water-soluble drugs could result from improved drug delivery, we quantitatively assessed pharmacokinetic factors in seven patients.

The capillary permeability surface product was found to be significantly increased in central nervous system lymphomas compared with glioblastoma multiforme, medulloblastomas, and metastases.

We hypothesized that HIOMT could serve as a tumor marker of PPTs, and we investigated HIOMT localization and HIOMT expression in samples of normal human tissue and in PPTs, primitive neuroectodermal tumors, and medulloblastomas.

The proportions of HIOMT-immunoreactive cells successively decreased in the following tumors: pineocytoma, pineal parenchymal tumor of intermediate differentiation, and pineoblastoma.

A few HIOMT-immunoreactive cells were observed in one of 6 primitive neuroectodermal tumors and 23 of 42 medulloblastomas.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] miR-124 is frequently down-regulated in medulloblastoma and is a negative regulator of SLC16A1.

Given that miR-124 is preferentially expressed in differentiating and mature neurons and external granule cells of cerebellum are thought to be cells-of-origins of medulloblastomas, we investigated if miR-124 played a role in the development of medulloblastomas.

Knockdown of SLC16A1 by siRNA induced cell death in medulloblastoma cells.

In conclusion, our study demonstrates that miR-124 deregulation is common in medulloblastomas, and restoration of its function inhibits cell proliferation, suggesting that miR-124 may act as a growth suppressor.

Our findings also raise the possibility that the miR-124/SLC16A1 pathway may represent a novel therapeutic target for treatment of malignant medulloblastomas.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

The amino acids glutamate and gamma-aminobutyric acid (GABA) have primarily been characterized as the most prevalent excitatory and inhibitory, respectively, neurotransmitters in the vertebrate central nervous system.

GABA, glutamate, and their complement of receptors are essential signaling molecules that regulate developmental processes in both embryonic and young adult mammals.

From our review of the literature and these data, we hypothesize that GABA(A) receptors and metabotropic glutamate receptors may be a novel target for the pharmacological regulation of the cerebellar tumors, medulloblastomas.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

The present study aimed to explore a potential of (123)I-MIBG to differentiate embryonal tumors from other types of brain tumors.

METHODS: Sixteen patients with brain tumors including three medulloblastomas, one neuroblastoma, six gliomas, and six meningiomas were examined with single-photon emission computerized tomography (SPECT) using (123)I-MIBG.

The (123)I-MIBG uptake of tumors was defined as the ratios of tumor/nontumor (early and delayed T/NT) on SPECT images scanned 30 min and 6 h after intravenous injection of the tracer, respectively.

RESULTS: The T/NT ratios on the early images for embryonal tumors (medulloblastomas and neuroblastoma), gliomas, and meningiomas were 3.2+/-1.7 (mean+/-SD), 1.4+/-0.3, and 1.6+/-0.5, respectively.

CONCLUSION: Early high accumulation and high retention on delayed imaging may indicate a possibility of (123)I-MIBG SPECT in differentiating embryonal brain tumors from gliomas and meningiomas.